Transplantation Unit, Massachusetts General Hospital and Harvard Medical School, Boston, MA.
Am J Transplant. 2013 Oct;13(10):2739-42. doi: 10.1111/ajt.12381. Epub 2013 Aug 5.
Type 1 primary hyperoxaluria (PH1) causes renal failure, for which isolated kidney transplantation (KT) is usually unsuccessful treatment due to early oxalate stone recurrence. Although hepatectomy and liver transplantation (LT) corrects PH1 enzymatic defect, simultaneous auxiliary partial liver transplantation (APLT) and KT have been suggested as an alternative approach. APLT advantages include preservation of the donor pool and retention of native liver function in the event of liver graft loss. However, APLT relative mass may be inadequate to correct the defect. We here report the first case of native portal vein embolization (PVE) to increase APLT to native liver mass ratio (APLT/NLM-R). Following initial combined APLT-KT, both allografts functioned well, but oxalate plasma levels did not normalize. We postulated the inadequate APLT/NLM-R could be corrected by trans-hepatic native PVE. The resulting increased APLT/NLM-R decreased serum oxalate to normal levels within 1 month following PVE. We conclude that persistently elevated oxalate levels after combined APLT-KT for PH1 treatment, results from inadequate relative functional capacity. This can be reversed by partial native PVE to decrease portal flow to the native liver. This approach might be applicable to other scenarios where partial grafts have been transplanted to replace native liver function.
1 型原发性高草酸尿症 (PH1) 可导致肾衰竭,孤立性肾移植 (KT) 通常因早期草酸结石复发而不成功。虽然肝切除术和肝移植 (LT) 可纠正 PH1 酶缺陷,但同时辅助性部分肝移植 (APLT) 和 KT 已被提议作为一种替代方法。APLT 的优点包括保留供体库和在肝移植物丢失的情况下保留供体肝脏的功能。然而,APLT 的相对质量可能不足以纠正缺陷。我们在此报告首例采用门脉栓塞术(PVE)增加 APLT 与供体肝质量比(APLT/NLM-R)的病例。在最初的联合 APLT-KT 后,两个同种异体移植物均功能良好,但草酸血浆水平未正常化。我们推测,通过经肝门 PVE 可纠正不足的 APLT/NLM-R。增加的 APLT/NLM-R 导致 PVE 后 1 个月内血清草酸降低至正常水平。我们得出结论,PH1 治疗中联合 APLT-KT 后持续升高的草酸水平是由于相对功能能力不足。通过部分门脉栓塞术减少向供体肝脏的门静脉血流可以逆转这种情况。这种方法可能适用于其他已移植部分移植物以替代供体肝脏功能的情况。